本文關(guān)鍵詞：狼瘡性腎炎足細(xì)胞病患者的臨床病理特征及預(yù)后　出處：《腎臟病與透析腎移植雜志》2015年04期 　論文類型：期刊論文

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【摘要】：目的:回顧性分析狼瘡性腎炎足細(xì)胞病(狼瘡足細(xì)胞病)患者的臨床、病理特征及遠(yuǎn)期預(yù)后。方法:系統(tǒng)性紅斑狼瘡(SLE)伴腎損害,經(jīng)腎活檢組織學(xué)和電鏡檢查符合狼瘡足細(xì)胞病的患者53例(女48例,男5例,中位年齡31歲,中位病程1.5月)。回顧性分析其臨床、病理特征及遠(yuǎn)期預(yù)后。結(jié)果:53例狼瘡足細(xì)胞病占同期狼瘡性腎炎的1.41%,其中50例臨床表現(xiàn)腎病綜合征,17例(32.1%)伴急性腎損傷(AKI),合并鏡下血尿和高血壓各9例(17.0%)。根據(jù)腎活檢光鏡改變分為系膜增生性病變(MP,n=31)、輕微病變(MCD,n=13)和局灶節(jié)段性腎小球硬化(FSGS,n=9)三組。電鏡觀察中位足細(xì)胞足突融合比例85%,三組間無(wú)明顯差異。FSGS組AKI發(fā)生率(77.8%)顯著高于MP組(22.6%)和MCD組(23.1%)(P0.01),腎小管間質(zhì)急性病變程度也明顯高于其他兩組(P0.05)。與MCD組(23.1%)相比,MP組(83.9%)和FSGS組(88.9%)低C3血癥的比例顯著升高(P0.01)。經(jīng)激素或激素聯(lián)合免疫抑制劑誘導(dǎo)治療后,69.8%獲得完全緩解,FSGS組完全緩解率(22.2%)顯著低于MCD組(92.3%)和MP組(74.2%)(P0.01)。中位隨訪時(shí)間60月,29例(54.7%)腎病復(fù)發(fā),13例復(fù)發(fā)后行重復(fù)腎活檢,其中6例發(fā)生病理轉(zhuǎn)型,無(wú)終末期腎病或死亡病例。結(jié)論:狼瘡足細(xì)胞病以腎病綜合征或伴AKI為主要特征,組織學(xué)改變可為MCD、MP或FSGS,激素或激素聯(lián)合免疫抑制劑治療敏感,FSGS者AKI發(fā)生率高、腎小管損傷重且治療緩解率低。該病復(fù)發(fā)率高,部分重復(fù)腎活檢可見(jiàn)病理轉(zhuǎn)型,長(zhǎng)期隨訪預(yù)后良好。
[Abstract]:Objective: to retrospectively analyze the clinical, pathological features and long-term prognosis of patients with lupus nephritis podocyte disease (lupus podocyte disease). Methods: systemic lupus erythematosus (SLE) with renal damage. Renal biopsy and electron microscopy were performed in 53 patients with lupus podocytosis (48 female, 5 male, median age 31 years). The median course of disease was 1.5 months. Results 53 cases of lupus podocytosis accounted for 1.41% of lupus nephritis in the same period, of which 50 cases showed nephrotic syndrome. There were 17 cases with acute renal injury, 9 cases with hematuria and 9 cases with hypertension. According to the light microscope changes of renal biopsy, MP was divided into Mesangial proliferative lesions. The ratio of median podocyte to foot process fusion was observed by electron microscope in the three groups: MCDN 13) and focal segmental glomerulosclerosis (FSGSN 9). The incidence of AKI in FSGS group (77.8%) was significantly higher than that in MP group (22.6%) and MCD group (23.1P 0.01). The degree of acute renal tubulointerstitial lesion was also significantly higher than that of the other two groups (P 0.05, compared with MCD group). The proportion of hypoC3 in MP group (83.9) and FSGS group (88.9) was significantly higher than that in control group (P 0.01). 69.8% achieved complete remission. The complete remission rate in FSGS group was significantly lower than that in MCD group (92. 3%) and in MP group 74.2% (P 0. 01). The median follow-up time was 60 months. 29 cases (54.7%) Nephropathy recurred in 13 cases and repeated renal biopsy was performed in 13 cases, of which 6 cases underwent pathological transformation. Conclusion: lupus podocyte disease is characterized by nephrotic syndrome or AKI, and histological changes may be MCDMP or FSGS. The incidence of AKI was high, the renal tubule injury was serious and the treatment remission rate was low in the patients with hormone or hormone combined immunosuppressive agents. The recurrence rate of the disease was high, and the pathological transformation was observed in some repeated renal biopsies. Long-term follow-up has a good prognosis.
【作者單位】： 南京大學(xué)醫(yī)學(xué)院附屬金陵醫(yī)院(南京軍區(qū)南京總醫(yī)院)腎臟科 國(guó)家腎臟疾病臨床醫(yī)學(xué)研究中心全軍腎臟病研究所;
【基金】：國(guó)家科技支撐計(jì)劃課題(2011BAI10B04,2013BAI09B04,2015BAI12B05) 江蘇省自然科學(xué)基金(BK20131326)
【分類號(hào)】：R593.242
【正文快照】： 狼瘡性腎炎(LN)的臨床表現(xiàn)和病理改變存在一定聯(lián)系,臨床表現(xiàn)為腎病綜合征(NS)的LN患者腎活檢病理通常見(jiàn)免疫復(fù)合物沉積于腎小球毛細(xì)血管袢[1-2]。但近年研究發(fā)現(xiàn),少數(shù)臨床表現(xiàn)為NS的LN患者腎活檢病理僅見(jiàn)免疫復(fù)合物沉積于腎小球系膜區(qū),電鏡下見(jiàn)腎小球足細(xì)胞足突廣泛融合,與微

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